The heart in polymyositis: a prospective evaluation of 26 patients.

Although cardiac abnormalities have been reported in patients with idiopathic polymyositis-dermatomyositis (PM), the nature and extent of these abnormalities have varied. The purpose of this study was to determine the prevalence and to obtain a better description of the spectrum of cardiac abnormalities in a cohort of patients with PM by use of a thorough noninvasive cardiac evaluation. Accordingly, we studied 26 patients with a history of PM and clinically inactive myositis (22 polymyositis, 4 dermatomyositis) between June 1984 and June 1991. Examination included history, physical examination, 12-lead electrocardiography, 24-h ambulatory electrocardiographic monitoring, chest radiography, transthoracic echocardiography, and radionuclide ventriculography. Of the patients studied, 77% were taking corticosteroid medications at a mean dose of 39 +/- 27 mg at the time of their evaluation. All 26 patients were identified as having two or more cardiac abnormalities. Cardiac symptoms and signs were common (62 and 81%, respectively), but were generally nonspecific. Electrocardiographic findings were most common (in 85% of cases), followed by findings on ambulatory monitoring (77%), echocardiography (42%), and radionuclide ventriculography (15%). The prevalence of mitral valve prolapse (8%) and hyperkinetic left ventricular contraction (12%) was significantly lower than previously reported. A secondary aim of this study was to determine associations between demographic variables including age, disease duration, cardiovascular symptoms, immunosuppressive therapy, autoantibody status, and creatinine phosphokinase level, and the presence of cardiac abnormalities. Of these patient variables, only increasing patient age was associated with an increased likelihood of cardiac abnormalities on noninvasive testing.(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary arterial ectasia: increased prevalence in patients with abdominal aortic aneurysm as compared to occlusive atherosclerotic peripheral vascular disease.

Coronary artery ectasia (CAE) is the saccular or fusiform dilatation of a coronary artery. CAE is found in 1.2% to 4.9% of patients at autopsy or during angiographic studies, with a similar prevalence of CAE found in patients with atherosclerotic peripheral vascular disease (PVD). Abdominal aortic aneurysm (AAA) and CAE are similar in pathogenesis and histology. To determine whether CAE occurs more frequently in patients with AAA than in occlusive forms of atherosclerotic PVD, a review of coronary angiograms was performed in patients who underwent cardiac catheterization and vascular reconstruction for AAA or occlusive atherosclerotic PVD of the lower extremities. Of 72 patients with AAA, 15 had CAE (20.8%) compared with only 2 of 69 patients with atherosclerotic PVD (2.9%) (p < 0.003). CAE was predominantly discrete, located in the left coronary system, and associated with significant coronary atherosclerosis. CAE may be more prevalent in patients with AAA resulting from a similar pathogenetic process.

Sudden cardiac death associated with isolated congenital coronary artery anomalies.

OBJECTIVES AND BACKGROUND: Congenital coronary anomalies are associated with sudden death and exercise-related death. Clarification of the risk and mechanisms of sudden death in patients with coronary anomalies may aid in decisions on intervention. METHODS: The clinicopathologic records of 242 patients with isolated coronary artery anomalies were reviewed for information on mode of death and abnormalities of the initial segment (acute angle takeoff, valvelike ridges or aortic intramural segments) and course of the anomalous coronary artery. RESULTS: Cardiac death occurred in 142 patients (59%); 78 (32%) of these deaths occurred suddenly. Of sudden deaths, 45% occurred with exercise. Sudden death (28 of 49, 57%) and exercise-related death (18 of 28, 64%) were most common with origin of the left main coronary artery from the right coronary sinus. Anomalous origin of the right coronary artery from the left coronary sinus was also commonly associated with exercise-related sudden death (6 of 13 sudden deaths, 46%). High risk anatomy involved abnormalities of the initial coronary artery segment or coursing of the anomalous artery between the pulmonary artery and aorta. Younger patients (less than or equal to 30 years old) were significantly more likely than older patients (greater than or equal to 30 years old) to die suddenly (62% vs. 12%, p = 0.0001) or during exercise (40% vs. 2%, p = 0.00001) despite their low frequency of significant atherosclerotic coronary artery disease (1% vs. 40%, p = 0.00001). CONCLUSIONS: Younger patients (less than or equal to 30 years old) with an isolated coronary artery anomaly are at risk of dying suddenly and with exercise. Therefore, greater effort for early detection and surgical repair of these lesions is warranted.

Current status of coronary artery operation in septuagenarians.

Previous reports of elderly patients undergoing coronary artery bypass grafting have not addressed the current era of aggressive percutaneous angioplasty and frequent urgent or emergent operation. To investigate this important subgroup of patients, we analyzed our recent coronary artery bypass grafting experience with patients 70 years of age or older. From January 1984 to January 1989, 121 consecutive patients in this age range underwent surgical revascularization at our institution. Overall in-hospital operative mortality (OM) was 7.4% (9/121), with 77.8% (7/9) of deaths due to cardiac causes. Serious postoperative morbidity occurred in 71.1% (86/121). Surgical priority was significantly correlated with operative mortality: for elective cases, the OM was 2.9% (2/68), but it was 8.6% (3/35) for urgent cases (p less than 0.05) and 22.2% (4/18) for emergency cases (p less than 0.05). Univariate analysis isolated the need for inotropic support, intraaortic balloon pump, reoperation, cardiopulmonary resuscitation, and emergency status as significant risk factors for OM (p less than 0.05). Multivariate stepwise logistic regression analysis identified the need for inotropic support, intravenous nitroglycerin, reoperative coronary artery bypass grafting, and hypertension as independently significant risk factors. A logistic risk equation developed from this population accurately modeled OM at the extremes of operative risk. Three (3.1%) of the 97 patients predicted to have less than 5% OM died, whereas all patients predicted by the model to have greater than 90% OM died. These results indicate that in spite of relatively high morbidity and mortality rates, elderly patients have a very acceptable operative risk in the current era of high-risk coronary artery bypass grafting. This is particularly true if elective revascularization is possible.

Anomalous origin and course of coronary arteries in adults: identification and improved imaging utilizing transesophageal echocardiography.

Anomalous origin of a coronary artery is rare, but may represent a clinically significant abnormality, since some anomalies are associated with myocardial ischemia, infarction, and sudden death. Diagnosis may elude routine screening procedures, and even when an anomalous vessel is identified angiographically, it may be difficult to delineate its true course on the basis of angiography alone. The purpose of this study was to determine whether transesophageal echocardiography (TEE) is of value in making the diagnosis and outlining the course of anomalous coronary arteries. Five adult patients with anomalous origin of a coronary artery were studied by monoplane TEE and selective coronary angiography. Transthoracic echocardiography (TTE) was also performed in four of these five patients. Anomalous coronary ostia were visualized in four of five patients utilizing TEE, but in none of four patients by TTE. A proximal segment of the anomalous coronary vessel was identified in all five patients by TEE, and in only two of four patients by TTE. TEE images were consistently of superior diagnostic quality. TEE is a valuable tool for the echocardiographic identification of anomalous coronary arteries, and is superior to TTE in adult patients.

Distribution of Lyb-4.1 and other membrane antigens on murine lymphoid tumors.

The distribution of membrane antigens on 6 DBA/2-derived tumors (L1210, L5178Y, P815, ABLS 11, ABLS 12, and ABLS 13) was studied by direct cytotoxicity and quantitative absorption assays. Lyb-4.1 antigen was found solely on the L1210 tumor. Iad antigens were absent from all tumors, and H-2Kd and H-2Dd antigens were present on all tumors. Immunoglobulin was adsorbed to the ascites tumors and lost after 3 days or more in tissue culture. These studies were performed to characterize the distribution of DBA/2 membrane antigens on DBA/2-derived tumors as a base line for functional and chemical studies with these tumors and with their solubilized proteins.

Internal synthesis of p23,30 by several lymphoid malignancies.

The aim of this study was to prove the internal synthesis of p23,30 antigen (HLA-D related determinant) on human leukemias and lymphomas on which it has been detected with complement-dependent cytotoxic assays. Murine Ia antigens similar to p23,30 antigen are found on many subsets of cells in the mouse (B lymphocytes, macrophages, allogeneically activated T lymphocytes) and on intercellularly transferred immunoregulatory molecules, which may be adsorbed to other cells. The question exists whether the p23,30 antigen, which occurs on a wide range of human leukemias, is internally synthesized by these tumors or, in some instances, is synthesized by normal lymphocytes and is adsorbed to the leukemic cells. The expression of p23,30 antigen on a limited series of human leukemias and lymphomas was detected by a complement dependent, cytotoxicity assay. The internal synthesis of p23,30 antigen and p44,12 (HLA-A and -B antigens and beta2-microglobulin) was confirmed by immunoprecipitation and these antigens from [35S]methionine labeled, detergent solubilized membranes of tumor cells. In each instance, the synthesis of p23,30 antigen by the malignant cells was confirmed. The distribution of p23,30 antigen (and 1a antigen) on subsets of normal cells and in immunoregulatory molecules was reviewed. In view of these findings, the role of p23,30 antigen in the diagnosis of subsets of human hematologic malignancies was reconsidered.

Heterogeneity in the membrane proteins of human lymphoid cell lines as seen in sodium dodecyl sulfate-polyacrylamide electrophoresis slab gels.

The proteins of [35S]methionine-labeled membranes of six human lymphoid cell lines were examined by electrophoresis in sodium dodecyl sulfate-polyacrylamide gradient slab gels in order to identify molecular differences among these tumors. The lymphoid cells were internally labeled with [35S]methionine, their membranes were isolated, and the reduced and alkylated membrane proteins were treated electrophoretically in sodium dodecyl sulfate-polyacrylamide gradient slab gels. The gel patterns of over 100 membrane proteins per cell were highly complex but reproducible and, in that sense, constituted fingerprints of the individual tumors. Several proteins occurred uniquely on one or a few tumors. Some protein bands were identified to be serologically recognized membrane antigens by electrophoresis of immunopurified antigen in parallel to membrane samples. p44,12, a complex of proteins with molecular weights of 44,000 and 12,000 (HLA-A and -B antigens and beta2-microglobulin), and p29,34, (HLA-D antigen) were identified in this manner. High-resolution sodium dodecyl sulfate-polyacrylamide gradient gel electrophoresis can be used to catalog and describe lymphocyte membrane proteins and perhaps to identify subsets of lymphoid cancers.